RESOLUTION OF HEART-FAILURE IN PATIENTS WITH AL AMYLOIDOSIS

Background: Patients with AL amyloidosis and congestive heart failure have a very poor prognosis. To date, the recovery of these patients ha s not been described in detail. Objective: To determine the frequency and characteristics of regression of disease in patients with congesti ve heart failure due to AL amyloidosis. Design: Review of patients wit h systemic AL amyloidosis. Setting: An international referral center f or amyloidosis in the United States. Patients: 140 patients with conge stive heart failure due to AL amyloidosis who were seen between 1983 a nd 1994. Measurements: Functional status, Doppler echocardiography, an d objective measurements of disease activity. Results: 3 of 140 patien ts (2.1%) had marked resolution of congestive heart failure and eviden ce for remission of disease activity. All 3 had been treated with melp halan. Conclusions: Melphalan appears to have had a favorable effect i n 3 patients with AL amyloidosis and heart failure. The abolition of l ight chains that was seen in these 3 patients suggests that light-chai n toxicity may play a role in the genesis of heart failure in patients with AL amyloidosis.